多系统朗格汉斯细胞组织细胞增生症一例

A case of multisystem Langerhans cell histiocytosis

17岁女性患者,全身散在红斑、部分破溃1年余。既往有甲状腺功能减退、肝功能异常、肺大疱、气胸、下丘脑磁共振成像示异常信号等病史。皮损组织病理:真皮浅中层可见弥漫致密的以组织样细胞为主的浸润。免疫组化染色示:S-100蛋白、Langerin、CD1a阳性,Ki67 30%阳性。诊断:朗格汉斯细胞组织细胞增生症。患者同时伴有重度混合性肺通气功能障碍、小气道中度陷闭、肾功能不全、电解质紊乱、贫血、凝血时间延长、骨密度降低等多系统累及征象。给予环磷酰胺+长春地辛+甲泼尼龙方案化疗,2个疗程治疗后,皮损部分消退,肝功能有所改善,但贫血症状加重,后放弃治疗。

A 17 years old female patient had been suffering from whole body rash accompanied with partial ulceration for almost one year. In the course of the disease, the patient was diagnosed with hypothyroidism, hepatic insufficiency, pulmonary bulla, pneumothorax and thalamic abnormal signals. Skin histopathology: diffuse and dense histocyte like cells could be seen in the superficial and middle layer of dermis. Immunohistochemistry: S100(+), langerin(+), CD1 a(+), Ki67 30%(+). Diagnosis: Langerhans cell histiocytosis. Further examination prompted the patient was also complicated by severe mixed lung ventilation dysfunction and moderate small airway closed, renal insufficiency, electrolyte disorders, anemia, prolong clotting time, reduced bone mineral density and so on. The patient was treated with Cyclophosphamide+Vindesine+Methylprednisolone chemotherapy.